Glinianaia Svetlana V, Embleton Nicholas D, Rankin Judith
Institute of Health and Society, Newcastle University, Newcastle upon Tyne, United Kingdom.
Birth Defects Res A Clin Mol Teratol. 2012 Jul;94(7):511-20. doi: 10.1002/bdra.23030. Epub 2012 Jun 22.
Few studies have assessed quality of life (QOL) for children born with major structural congenital anomalies. We aimed to review studies reporting QOL in children and adults born with selected congenital anomalies involving the digestive system.
Systematic review methods were applied to literature searches, development of the data extraction protocol, and the review process. We included studies published in English (1990-2010), which used validated instruments to assess QOL in individuals born with congenital diaphragmatic hernia, esophageal atresia, duodenal atresia or abdominal wall defects.
Of 200 papers identified through literature searches, 111 were excluded after applying restrictions and removing duplicates. After scanning 89 abstracts, 32 full-text papers were reviewed (none on duodenal atresia), of which 18 (nine in children or adolescents and nine in adults) were included. Studies measured health-related QOL, but did not assess subjective wellbeing. Instruments used to assess health-related QOL in children varied considerably. In adults most studies used the Short Form 36. Many studies had methodological limitations, such as being from a single institution, retrospective cohorts, and low sample size. The summarized evidence suggests that health-related QOL of these children is affected by associated anomalies and ongoing morbidity resulting in lower physical functioning and general health perception. In adults, health-related QOL is comparable with the general population.
The reviewed studies considered health status and functioning as a major determinant of QOL. More studies assessing QOL in patients with major congenital anomalies are needed, and those involving children should use age-adjusted, validated instruments to measure both health-related QOL and self-reported subjective wellbeing.
很少有研究评估患有严重先天性结构异常的儿童的生活质量（QOL）。我们旨在回顾报告患有特定先天性消化系统异常的儿童和成人生活质量的研究。
系统评价方法应用于文献检索、数据提取方案的制定以及评价过程。我们纳入了1990年至2010年以英文发表的研究，这些研究使用经过验证的工具来评估患有先天性膈疝、食管闭锁、十二指肠闭锁或腹壁缺损的个体的生活质量。
通过文献检索确定的200篇论文中，在应用限制条件并去除重复项后，排除了111篇。在浏览89篇摘要后，对32篇全文论文进行了评审（无关于十二指肠闭锁的论文），其中18篇（9篇关于儿童或青少年，9篇关于成人）被纳入。研究测量了与健康相关的生活质量，但未评估主观幸福感。用于评估儿童与健康相关生活质量的工具差异很大。在成人中，大多数研究使用简短健康调查问卷（Short Form 36）。许多研究存在方法学局限性，例如来自单一机构、回顾性队列研究以及样本量较小。汇总的证据表明，这些儿童与健康相关的生活质量受到相关异常和持续发病的影响，导致身体功能和总体健康感知较低。在成人中，与健康相关的生活质量与一般人群相当。
所回顾的研究认为健康状况和功能是生活质量的主要决定因素。需要更多评估患有主要先天性异常患者生活质量的研究，并且涉及儿童的研究应使用经过年龄调整的、经过验证的工具来测量与健康相关的生活质量和自我报告的主观幸福感。
